Ontology highlight
ABSTRACT:
SUBMITTER: Egesipe AL
PROVIDER: S-EPMC5515002 | biostudies-literature | 2016
REPOSITORIES: biostudies-literature
Egesipe Anne-Laure AL Blondel Sophie S Lo Cicero Alessandra A Jaskowiak Anne-Laure AL Navarro Claire C Sandre-Giovannoli Annachiara De A Levy Nicolas N Peschanski Marc M Nissan Xavier X
NPJ aging and mechanisms of disease 20161110
Hutchinson-Gilford progeria syndrome (HGPS) is a rare genetic disorder that causes systemic accelerated aging in children. This syndrome is due to a mutation in the <i>LMNA</i> gene that leads to the production of a truncated and toxic form of lamin A called progerin. Because the balance between the A-type lamins is controlled by the RNA-binding protein SRSF1, we have hypothesized that its inhibition may have therapeutic effects for HGPS. For this purpose, we evaluated the antidiabetic drug metf ...[more]